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Natural history of a medulloblastoma: 30 months of wait and see in a child with a cerebellar incidentaloma


Zeilhofer, Ulrike B; Scheer, Ianina; Warmuth-Metz, Monika; Rushing, Elisabeth J; Pietsch, Torsten; Boltshauser, Eugen; Grotzer, Michael A; Gerber, Nicolas U (2013). Natural history of a medulloblastoma: 30 months of wait and see in a child with a cerebellar incidentaloma. Child's Nervous System, 29(7):1207-1210.

Abstract

INTRODUCTION: With the increasing use of neuroimaging studies, the discovery of incidental neoplastic lesions is becoming more frequent. However, standard procedures are lacking, and little is known about their optimal management. CASE REPORT: We here present the case of a boy with a cerebellar mass incidentally discovered on a CT scan performed after head trauma. In another scan performed after another incident of head trauma 14 months earlier, the lesion could be seen after retrospective examination. In view of the asymptomatic clinical and stable radiological status and the presumed diagnosis of a low-grade glioma, a watch-and-wait strategy was elected. After clinical and radiological progression was observed, the tumour was resected, 2½ years after the initial imaging study. Histological evaluation revealed a WNT pathway-activated classical medulloblastoma. DISCUSSION: To our knowledge, this is the first description of such a long natural history and pre-symptomatic period of a medulloblastoma. The long period of stability followed by a period of accelerated tumour growth is compatible with increasing biological aggressiveness, possibly related to the stepwise accumulation of genetic changes.

INTRODUCTION: With the increasing use of neuroimaging studies, the discovery of incidental neoplastic lesions is becoming more frequent. However, standard procedures are lacking, and little is known about their optimal management. CASE REPORT: We here present the case of a boy with a cerebellar mass incidentally discovered on a CT scan performed after head trauma. In another scan performed after another incident of head trauma 14 months earlier, the lesion could be seen after retrospective examination. In view of the asymptomatic clinical and stable radiological status and the presumed diagnosis of a low-grade glioma, a watch-and-wait strategy was elected. After clinical and radiological progression was observed, the tumour was resected, 2½ years after the initial imaging study. Histological evaluation revealed a WNT pathway-activated classical medulloblastoma. DISCUSSION: To our knowledge, this is the first description of such a long natural history and pre-symptomatic period of a medulloblastoma. The long period of stability followed by a period of accelerated tumour growth is compatible with increasing biological aggressiveness, possibly related to the stepwise accumulation of genetic changes.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Children's Hospital Zurich > Medical Clinic
04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:16 March 2013
Deposited On:11 Oct 2013 16:06
Last Modified:05 Apr 2016 17:01
Publisher:Springer
ISSN:0256-7040
Publisher DOI:https://doi.org/10.1007/s00381-013-2077-9
PubMed ID:23503614
Permanent URL: https://doi.org/10.5167/uzh-81611

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